Hemochromatosis

Hemochromatosis

Many chronic diseases are adversely affected by moderate to significant levels of iron overload. Excess iron can accelerate the aging process by catalyzing the production of free radicals that cause harmful oxidative stress leading to cell damage, lipid peroxidation and DNA mutagenesis.

A severe iron overload could lead to hemochromatosis without the presence of other disease factors. Lesser levels of iron overload, even in individuals whose ferritin and/or transferrin saturation levels range only toward the upper end of normal laboratory ranges, can result in free (or non-transferrin bound) iron that will catalyze the cycling of free radicals resulting in tissue damage often associated with chronic diseases. The iron-mediated disease process is associated with iron levels well below those observed in hemochromatosis and has been implicated in multiple endocrinopathies, potentially lethal hepatic and or cardiac complications.

Iron-chelating therapy with deferoxamine reduces iron excess and dramatically prolongs survival.

Ophthalmologic and audiologic evaluations are recommended when on chelation therapy. Zinc deficiency may be a result of chelation. As vitamin C levels may be low, replacement of it should be with care since vitamin C increases the toxicity of iron.

Last modified
13 October 2019
ATC

V03AC01 Deferoxamine

 

Desferal

V03AC03 Deferasirox

 

Exjade

V03AC02 Deferipron

Ferriprox

LOINC

20567-4 Ferritin in Serum or Plasma

Units: mass/​volume (µg/L)

ICD

E83.111 Hemochromatosis due to repeated red cell blood transfusions

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General Medical Guideline